医学
美罗华
显微镜下多血管炎
肉芽肿伴多发性血管炎
血管炎
内科学
重症监护医学
疾病
淋巴瘤
作者
Baptiste Chevet,Divi Cornec,Marta Casal Moura,Émilie Cornec-Le Gall,Fernando C. Fervenza,Kenneth J. Warrington,Ulrich Specks,Alvise Berti
出处
期刊:Rheumatology
[Oxford University Press]
日期:2022-10-31
卷期号:62 (5): 1787-1803
被引量:9
标识
DOI:10.1093/rheumatology/keac623
摘要
ANCA-associated vasculitides (AAV) are a group of rare, primary, systemic necrotizing small-vessel vasculitides. Granulomatosis with polyangiitis and microscopic polyangiitis account for ∼80-90% of all AAV. Exposure to silica dust, farming and chronic nasal Staphylococcus aureus carriage are associated with increased risk of developing AAV. When a diagnosis of AAV is suspected, as in patients with multisystem organ dysfunction or those with features such as chronic recurrent rhinosinusitis, cavitated lung nodules, palpable purpura or acute kidney injury, then appropriate further investigations are needed, including ANCA testing. In this scenario, a structured clinical assessment should be conducted, evaluating all the organs possibly involved, and tissue biopsy may be necessary for confirmation of the diagnosis. Therapeutic algorithms vary based on the severity of AAV, the clinical diagnosis/ANCA specificity, and the patient's age, weight, comorbidities and prognosis. Recent data favour rituximab as a preferable option for both induction and maintenance of remission. In addition, regimens with less glucocorticoids are equally effective and safer in inducing remission compared with conventional regimens, and avacopan is an effective glucocorticoid-sparing option. In contrast, there is not compelling evidence to support the routine use of plasma exchange in addition to standard remission-induction therapy in AAV. ANCA and other biomarkers can be helpful in association with clinical assessment to guide diagnosis and treatment decisions. Patients should be frequently evaluated during follow-up for possible disease relapses or treatment-related morbidity, and for monitoring damage accrual, especially metabolic and cardiovascular damage.
科研通智能强力驱动
Strongly Powered by AbleSci AI